Sjogrens

Question 1

Confirming Dx of Sjogren’s? (4)

Answer 1

1. Schirmer test (test for dry eyes): filter paper applied to margin of lower eyelid → wetting <5mm
2. Salivary gland hypofunction - e.g. scintigraphy or sialometry
3. Anti Ro/SSA and La/SSB
4. If dx unclear, consider Salivary gland biopsy (look for extensive lymphocytic infiltration with glandular/ductal atrophy)

Question 2

What are the extra-glandular features of Sjogren’s syndrome? (5)

Answer 2

Most important for long case

1. ILD (NSIP, UIP, COP)
2. Renal (most common = tunulo-interstitial nephritis, 25-50%)
3. Transverse myelitis or NMO (many have Aquaporin Abs)
4. Peripheral neuropathy
5. NHL (x40 risk) - and other cytopaenias

Others include

Skin: Xerosis (abnormal dryness), cutaneous vasculitis

Arthralgia & arthritis

GIT/Biliary - dysphagia, nausea, celiac

Heart: CV disease

Question 3

What is the most common cause of secondary Sjogren’s? (1)

Question 4

Non-pharmacological Mx of Sjogren’s?

Answer 4

1. Educate: self-care of sicca symptoms [eye/mouth] is the key.
   - Artificial tears: regular
   - Tear conservation: avoid windy/dry outdoors, use humidifiers, wraparound sunglasses
   - Artificial saliva (pilocarpine in resistant cases), oral hyginen, hydration, avoid sugary fluids, carbonated drinks, regular dental appointments

So regular dental + ophthal F/U

2. Immunisation
3. Smoking cessation (worsens dry mouth, inc risk of CVD, diminishes efficacy of HCQ)
4. Diet and nutrition: dietry FA supplements - omega-3
5. Counsel on Pregnancy (risk of congenital heart block) - refer to perinatologist & surveillance for CHD

Question 5

Pharmacological Mx of Sjogren’s - general approach?

Answer 5

For sicca symptoms, generally does not require systemic ImmSx.

Indicated in organ-based disease + consititutional symptoms - skin rashes, arthritis, pulmonary, renal.

Approach is similar to that of SLE and RA

1. Steroids - severe glandular or extraglandular disease
2. Hydroxychloroquine
3. DMARDs: MTX, Leflunomide, AZA, sulfasalazine, MMF
4. ImmSx: CsA, Tac, Cyclophosphamide, Rituximab.

Question 6

Prognosis of Sjogren’s?

Answer 6

Extraglandular involvement is associated with a reduced quality of life, and some evidence has suggested that Sjögren’s syndrome (SS) is associated with increased mortality compared with matched controls.